Newly identified autoantibodies: relationship to idiopathic inflammatory myopathy subsets and pathogenesis.
OBJECTIVE
There is increasing evidence that patients with polymyositis and dermatomyositis have specific clinico-serological profiles. Myositis-specific autoantibodies target intracellular proteins involved in key processes such as translocation and nuclear transcription, and are closely linked to patterns of disease expression. This review highlights the recent work on novel myositis-specific autoantibodies, their autoantigen targets, the relationship to clinical subsets and potential role in pathogenesis.


RESULTS
During the annual review period novel autoantibodies have been described in adult-onset dermatomyositis (anti-SAE autoantibodies) and juvenile dermatomyositis (anti-p155/140 autoantibodies) (anti-MJ or anti-p140 autoantibodies). These findings are important because the function of the autoantigen targets may point to shared pathogenic pathways. Studies highlighting the potential role of autoantigen-driven responses and autoantibody production in disease initiation and propagation are discussed, as well as further evidence on the relationship between autoantigens and corresponding target tissues.


CONCLUSIONS
Considerable progress has been made emphasizing the striking association between genotype, serotype and clinical phenotype in the myositis disease spectrum. Further characterization of the identity and function of autoantigen systems in target tissues will greatly facilitate investigation of models of autoimmunity in this disease.
Gunawardena
Harsha
H
Department of Rheumatology, Royal National Hospital for Rheumatic Diseases, Bath Institute for Rheumatic Diseases and School for Health, University of Bath, Bath, UK.
Betteridge
Zoe E
ZE
McHugh
Neil J
NJ
D000328:Adult; D000918:Antibody Specificity; D001323:Autoantibodies / Q000378:metabolism*; D001324:Autoantigens; D002648:Child; D003882:Dermatomyositis / Q000209:etiology / Q000276:immunology; D006801:Humans; D036341:Intercellular Signaling Peptides and Proteins; D009220:Myositis / Q000209:etiology* / Q000276:immunology*; D010455:Peptides / Q000276:immunology; D044764:Ubiquitin-Activating Enzymes / Q000276:immunology
D001323:Autoantibodies; D001324:Autoantigens; C501597:CADM-140 peptide, human; D036341:Intercellular Signaling Peptides and Proteins; D010455:Peptides; D044764:Ubiquitin-Activating Enzymes